Mikulicz syndrome is a chronic condition characterized by the abnormal enlargement of glands in the head and neck, including those near the ears (parotids) and those around the eyes (lacrimal) and mouth (salivary). The tonsils and other glands in the soft tissue of the face and neck may also be involved. Although the disorder is almost always described as benign, it always occurs in association with another underlying disorder such as tuberculosis, leukemia, syphilis, Hodgkin’s disease, lymphosarcoma, Sjögren syndrome, or lupus (SLE). People who have Mikulicz syndrome are at heightened risk for developing lymphomas.
Some people with Mikulicz syndrome may experience recurring fevers. The fever may be accompanied by dry eyes, diminished tear production (lacrimation), and inflammation of various parts of the eyes (uveitis). Lacrimal gland enlargement, parotid gland enlargement, dry mouth and dry eyes are the classic signs.
The exact cause of Mikulicz syndrome is not known. Some scientists believe that Mikulicz syndrome should be considered a form of Sjögren syndrome.
Mikulicz syndrome is characterized by the sudden onset of extreme dryness in the mouth (xerostomia) that may lead to difficulty swallowing and tooth decay. Other symptoms include enlarged tear glands (lacrimal glands), leading to absent or decreased tears; enlarged glands in the neck (parotid glands); hard, painless swellings (tumefactions) of the saliva glands (salivary glands) of the mouth and those near the ears (parotid). Glands near the jaw (submaxillary) may also become swollen. Symptoms may persist for long periods of time or come and go with frequent recurrences. The symptoms of Mikulicz syndrome are very similar to those of Sjögren syndrome and some researchers suspect that they may be the same disorder (see Related Disorders section of this report).
The exact cause of Mikulicz syndrome is not known, although it is suspected to be an autoimmune disorder. Autoimmune disorders are caused when the body’s natural defenses against “foreign” or invading organisms (e.g., antibodies) begin to attack healthy tissue for unknown reasons.
The symptoms of Mikulicz syndrome may occur due to the excessive accumulation of certain white blood cells (lymphocytes) into many glands of the face, mouth, and/or neck.
Mikulicz syndrome affects more females than males and most often presents during the middle adult years.
It often occurs in combination with Sjögren syndrome. Some scientists have speculated that Mikulicz Syndrome and Sjögren Syndrome may actually be the same disorder.
Biopsy of one of the swollen glands is key to the diagnosis of Mikulicz syndrome. An ultrasound examination of the area may help to rule out other reasons for gland swelling. Treatment of this disorder is symptomatic. Medical therapies are more productively directed toward the treatment of any underlying disease. Artificial tears may be used to maintain moisture in the eyes, and artificial saliva may be used to treat oral symptoms.
Some individuals with Mikulicz syndrome may be instructed to follow a soft moist diet. This may help to reduce the pain caused by chewing and swallowing. Other treatment is symptomatic and supportive.
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Leung AKC. Benign Lymphoepithelial Lesion. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:17.
Fox RI, Kang H-I. Sjögren’s Syndrome. In: Kelley WN, Harris ED, Ruddy S, et al. Textbook of Rheumatology. 4th ed. W. B. Saunders Company. Philadelphia, PA; 1993:931-41.
Hochberg MC. Sjögren’s Syndrome. In: Bennett JC, Plum F. Eds. Cecil Textbook of Medicine. 20th ed. W.B. Saunders Co., Philadelphia, PA; 1996:1488-90.
Goto TK, Shimizu M, Kobayashi I, et al. Lymphoepithelial lesion of the parotid gland. Dentomaxillofac Radiol. 2002;31:198-203.
Sato K, Kawana M, Sato Y, et al. Malignant lymphoma in the head and neck associated with benign lymphoepithelial lesion of the parotid gland. Auris Nasus Larynx. 2002;29:209-14.
Miracco C, Schurfeld K, Cardone C, et al. Benign lymphoepithelial lesion associated with squamous cell carcinoma of the skin: an immunohistochemical and molecular genetic study. J Cutan Pathol. 2002;29:33-37.
Amin MA, Bailey BM, Patel SR. Clinical and radiological evidence to support superficial parotid ectomy as the treatment of choice for chronic parotid sialadenitis: a retrospective study. Br J Oral Maxillofac Surg. 2001;39:348-52.
Carbone A, Gloghini A, Ferlito A. Pathological features of lymphoid proliferations of the salivary glands: lymphoepithelial sialadenitis versus low-grade B-cell lymphoma of the malt type. Ann Otol Rhinol Laryngol. 2000;109(12 Pt1):1170-75.
Tsubota K, Fujita H, Tsuzaka K, et al. Mikulicz’s disease and Sjögren’s syndrome. Invest Ophthalmol Vis Sci. 2000;41:1666-73.
Ihrler S, Baretton GB, Menauer F, et al. Sjögren’s syndrome and MALT lymphomas of the salivary glands: a DNA-cytometric and interphase-cytogenetic study. Mod Pathol. 2000;13:4-12.
Ihrler S, Zietz C, Sendelhofert A, et al. Lymphoepithelial duct lesions in Sjögren-type sialadenitis. Virchows Arch. 1999;434:315-23.
Leung AK, Wong AL, Robson WL, et al. Benign lymphoepithelial lesion (Mikulicz’s syndrome) of the submandibular glands in a four-year-old boy. Otolyryngol Head Neck Surg. 1994;111(3 Pt1):302-04.
FROM THE INTERNET
Fox RI, Michelson PE, Frosio D. Sjögren’s Syndrome: A Guide for the Patient. Revised Version; 8.16.02. 76pp.
HONselect. Mikulicz’ Disease. Last modified: Jan 29 2003. 2pp.
GPnotebook. Mikulicz syndrome. nd. 1p.
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